背景:罕见原发性恶性骨肉瘤(RPMBS)占原发性高级别骨肿瘤的5%-10%,是主要的治疗挑战。介绍了参加欧洲骨40以上肉瘤研究(EURO-B.O.S.S.)的RPMBS患者的结局。
方法:纳入标准如下:年龄41至65岁,诊断为高级梭形细胞,多形性,或血管RPMBS。化疗方案包括阿霉素60mg/m2,异环磷酰胺9g/m2,和顺铂90mg/m2;在组织学反应不佳的情况下,术后添加甲氨蝶呤8g/m2。不良事件通用术语标准2.0版,卡普兰-迈耶曲线,对数秩测试,并使用单变量Cox回归模型。
结果:总计,113名患者可进行评估分析。患者年龄中位数为52岁(范围,40-66岁),67名患者为男性。88例肿瘤被归类为未分化多形性肉瘤(UPS),20人被归类为平滑肌肉瘤,三个被归类为纤维肉瘤,和两个被归类为血管肉瘤。113个肿瘤中有83个位于四肢。113例患者中有95例没有转移的证据。经过6.8年的中位随访(四分位数间距[IQR],3.5-9.8年),局限性疾病患者的5年总生存率为68.4%(IQR,56.9%-77.5%),为71.7%(IQR,58.1%-81.6%)的UPS患者和54.9%(IQR,平滑肌肉瘤患者的29.5%-74.5%)。81%的患者报告有III-IV级血液学毒性;23%的患者有II-III级神经毒性,和37.5%有I-II级肾毒性。局部疾病患者的5年总生存率明显较好,对于获得手术完全缓解的患者,当原发肿瘤位于四肢时。
结论:本系列中RPMBS患者的生存率与年龄匹配的高级别骨肉瘤患者的生存率相似。RPMBS患者可能会建议进行骨肉瘤样化疗。
Rare primary malignant bone sarcomas (RPMBS) account for 5%-10% of primary high-grade bone tumors and represent a major treatment challenge. The outcome of patients with RPMBS enrolled in the EUROpean Bone Over 40 Sarcoma Study (EURO-B.O.S.S) is presented.
Inclusion criteria were as follows: age from 41 to 65 years and a diagnosis of high-grade spindle cell, pleomorphic, or vascular RPMBS. The chemotherapy regimen included doxorubicin 60 mg/m2 , ifosfamide 9 g/m2 , and cisplatin 90 mg/m2 ; postoperative methotrexate 8 g/m2 was added in case of a poor histologic response. Version 2.0 of the Common Terminology Criteria for Adverse Events, Kaplan-Meier curves, log-rank tests, and univariate Cox regression models were used.
In total, 113 patients were evaluable for analysis. The median patient age was 52 years (range, 40-66 years), and 67 patients were men. Eighty-eight tumors were categorized as undifferentiated pleomorphic sarcomas (UPS), 20 were categorized as leiomyosarcomas, three were categorized as fibrosarcomas, and two were categorized as angiosarcomas. Eighty-three of 113 tumors were located in the extremities. Ninety-five of 113 patients presented with no evidence of metastases. After a median follow-up of 6.8 years (interquartile range [IQR], 3.5-9.8 years), the 5-year overall survival rate for patients with localized disease was 68.4% (IQR, 56.9%-77.5%), and it was 71.7% (IQR, 58.1%-81.6%) for patients with UPS and 54.9% (IQR, 29.5%-74.5%) for patients with leiomyosarcoma. Grade III-IV hematologic toxicity was reported in 81% patients; 23% had grade II-III neurotoxicity, and 37.5% had grade I-II nephrotoxicity. Five-year overall survival was significantly better for patients with localized disease, for patients who obtained surgical complete remission, and when the primary tumor was located in the extremities.
The survival of patients who had RPMBS in the current series was similar to that of age-matched patients who had high-grade osteosarcoma treated according to the same protocol. An osteosarcoma-like chemotherapy may be proposed in patients who have RPMBS.